Tomographic assessment of the spine in children with spondylocostal dysotosis syndrome

Abstract

Objective: The aim of this study was to perform a detailed tomographic analysis of the skull base, craniocervical junction, and the entire spine in seven patients with spondylocostal dysostosis syndrome.

Method: Detailed scanning images have been organized in accordance with the most prominent clinical pathology. The reasons behind plagiocephaly, torticollis, short immobile neck, scoliosis and rigid back have been detected. Radiographic documentation was insufficient modality.

Results: Detailed computed tomography scans provided excellent delineation of the osseous abnormality pattern in our patients.

Conclusion: This article throws light on the most serious osseous manifestations of spondylocostal dysostosissyndrome.

Figure 1

(a,b,c) Anteroposterior spine radiographs of three patients (a,b) are male patients of 5 and 7 years and c is a female patient of 9 years. All showed extensive malsegmentation of the entire spine (puzzle-like vertebrae) associated with ill-defined cervical spine abnormalities and asymmetry of the thoracic malformation and a variety of different intrinsic rib anomalies including partial fusion, broadening, and bifid ribs.

Figure 2

3D reconstruction CT scan of a-3-year old male patient with SCD showed skull base asymmetry associated with significant endocranial synchondrosis and defective ossification. The asymmetry is more marked in the middle and the posterior cranial fossa while the anterior cranial fossa is slightly asymmetric.

Figure 3

A 3 D reconstruction CT scan showed skull asymmetry in a 14-year-old patient (deformational plagiocephaly) without synostosis. A 3D reconstruction CT scan of the endocranial base showed asymmetric 3D position of the external acoustic meatus. The asymmetry is more marked in the middle and the posterior cranial fossa while the anterior cranial fossa was slightly asymmetric.

Figure 4

Coronal reformatted CT scan of a 7-year-old girl showed neurocentral synchondrosis and a pseudo-Jefferson fracture of the lateral mass (arrow).

Figure 5

A 3D reconstruction CT scan of the same patient showed posterior arch rachischisis.

Figure 6

An axial reformatted CT scan of a 13-year- old- boy showed assimilation of the posterior arch of the atlas, a splitting deformity of the anterior arch and agenesis of the odontoid process.

Figure 7

A coronal 3DCT scan in a 3-year-old boy showed massive malsegmentation throughout the entire cervical spine anteriorly, with smooth outlines to the vertebral bodies, the so-called “pebble beach sign”.

Figure 8

A 3 D reformatted CT scan showed Spin bifida occulta and extensive malsegmentation along the posterior spine elements are associated with bilateral rib intrinsic anomalies including partial fusion, broadening, and bifid rib in the same child.

Figure 9

A 3 D reconstruction CT scan of the lumbar spine in a 15-year-old boy showed a unilateral, posterior unsegmented bar including the upper three lumbar vertebrae associated with fusion of the corresponding spinous processes.

Figure 10

A 3D reconstruction CT scan of a 9-year-old girl showed extensive failure of the fusion of the spinous processes of the lumbar vertebrae associated with malsegmentation.